The ZIC gene family in development and disease

Clin Genet. 2005 Apr;67(4):290-6. doi: 10.1111/j.1399-0004.2005.00418.x.

Abstract

The human ZIC gene family is comprised of five members encoding zinc-finger transcription factors, which are the vertebrate homologs of the Drosophila odd-paired gene. Mutations in ZIC genes in humans have recently been implicated in a wide variety of congenital malformations, including Dandy-Walker malformation, holoprosencephaly, neural tube defects, and heterotaxy. Mutant analysis of these genes in mice has underscored the conserved developmental roles of these genes. Further, this analysis has begun to elucidate the molecular and developmental mechanisms underlying these important birth defects.

Publication types

  • Review

MeSH terms

  • Animals
  • Carrier Proteins / genetics
  • DNA-Binding Proteins
  • Dandy-Walker Syndrome / genetics*
  • Functional Laterality / genetics
  • Gene Deletion
  • Gene Expression Regulation, Developmental*
  • Heart Defects, Congenital / genetics
  • Holoprosencephaly / genetics*
  • Homeodomain Proteins / genetics
  • Humans
  • Mice
  • Mutation
  • Nerve Tissue Proteins / genetics
  • Neural Tube Defects / genetics*
  • Nuclear Proteins
  • Transcription Factors / genetics*
  • Zinc Fingers / genetics*

Substances

  • Carrier Proteins
  • DNA-Binding Proteins
  • Homeodomain Proteins
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • Transcription Factors
  • ZIC1 protein, human
  • ZIC2 protein, human
  • ZIC3 protein, human
  • ZIC4 protein, human
  • ZIC5 protein, human